RT Journal Article
SR Electronic
T1 Familial cerebral amyloidosis and spongiform encephalopathy.
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 37
OP 45
DO 10.1136/jnnp.45.1.37
VO 45
IS 1
A1 Adam, J
A1 Crow, T J
A1 Duchen, L W
A1 Scaravilli, F
A1 Spokes, E
YR 1982
UL http://jnnp.bmj.com/content/45/1/37.abstract
AB Clinical and neuropathological investigations are presented of the "W" family in which there is a dominant inheritance of slowly progressive cerebellar ataxia and dementia. The disease is of insidious onset and its average duration more than 4 years. Pathological findings included amyloid deposition in cerebellar and cerebral tissue; vascular amyloid in one case; and spongiform encephalopathy and astrocytic hyperplasia typical of Creutzfeldt-Jakob disease. Neuritic plaques of the senile (Alzheimer) type were present to a lesser extent. This study confirms the familial association between cerebral amyloidosis and spongiform encephalopathy.